Introduction: Paragangliomas are rare tumors derived from neural crest cells, which are mostly localized in the adrenal medulla. Only 5-10% occur in extra-adrenal localizations. Given the potential of the paraganglioma to secrete catecholamines, some patients present constitutional symptoms such as headaches, tremors and hypertension. However, most patients are asymptomatic. Preoperative diagnosis of paraganglioma in asymptomatic patients is difficult but should be included in the differential diagnosis of solid mesenteric tumors. The gold standard therapy is complete resection, with the need for a long-term followup after surgery. Case Report: Our case study takes a look at a 32-year-old nulliparous woman observed in the emergency room with a severe low abdominal pain. The ultrasonography and computerized tomography revealed a large pelvic tumor with apparent origin in the left ovary. During an exploratory laparotomy, an encapsulated, well vascularized mass was found in the mesentery of the ileum, behind the uterus, conditioning the twisting and consequent bottlenecks of 30 cm of the small intestine. An enterectomy was performed